Excludes1

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan's syndrome (Q87.4)
  • 5-alpha-reductase deficiency (E29.1)

Excludes2

  • Ehlers-Danlos syndromes (Q79.6-)
  1. E76 Disorders of glycosaminoglycan metabolism

    1. E76.0 Mucopolysaccharidosis, type I

      1. E76.01 Hurler's syndrome

      2. E76.02 Hurler-Scheie syndrome

      3. E76.03 Scheie's syndrome

    2. E76.1 Mucopolysaccharidosis, type II

      Inclusion term(s):

      • Hunter's syndrome
    3. E76.2 Other mucopolysaccharidoses

      1. E76.21 Morquio mucopolysaccharidoses

        • E76.210 Morquio A mucopolysaccharidoses

          Inclusion term(s):

          • Classic Morquio syndrome
          • Morquio syndrome A
          • Mucopolysaccharidosis, type IVA
        • E76.211 Morquio B mucopolysaccharidoses

          Inclusion term(s):

          • Morquio-like mucopolysaccharidoses
          • Morquio-like syndrome
          • Morquio syndrome B
          • Mucopolysaccharidosis, type IVB
        • E76.219 Morquio mucopolysaccharidoses, unspecified

          Inclusion term(s):

          • Morquio syndrome
          • Mucopolysaccharidosis, type IV
      2. E76.22 Sanfilippo mucopolysaccharidoses

        Inclusion term(s):

        • Mucopolysaccharidosis, type III (A) (B) (C) (D)
        • Sanfilippo A syndrome
        • Sanfilippo B syndrome
        • Sanfilippo C syndrome
        • Sanfilippo D syndrome
      3. E76.29 Other mucopolysaccharidoses

        Inclusion term(s):

        • beta-Glucuronidase deficiency
        • Maroteaux-Lamy (mild) (severe) syndrome
        • Mucopolysaccharidosis, types VI, VII
    4. E76.3 Mucopolysaccharidosis, unspecified

    5. E76.8 Other disorders of glucosaminoglycan metabolism

    6. E76.9 Glucosaminoglycan metabolism disorder, unspecified

Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.